A team lead by Dr. Maj (Rtd) M. Quamrul Islam has recently started cone repair surgery to treat Ebstein's anomaly at the National Heart Foundation Hospital & Research Institute in Dhaka, Bangladesh.

What is Ebstein's anomaly?

Ebstein's anomaly is a rare heart defect that is present at birth (congenital). In Ebstein's anomaly, tricuspid valve — the valve between the chambers on the right side of the heart — does not work properly. Blood leaks back through the valve, making the heart work less efficiently. Ebstein's anomaly may also lead to enlargement of the heart or heart failure.

Symptoms:

Mild forms of Ebstein's anomaly may not cause symptoms until later in adulthood. If signs and symptoms are present, they may include shortness of breath, especially with exertion, fatigue, heart palpitations or abnormal heart rhythms (arrhythmias), a bluish discoloration of the lips and skin caused by low oxygen (cyanosis).

When to see a doctor

If you or your child has signs or symptoms of heart failure — such as feeling easily fatigued or short of breath, even with normal activity — or is showing blue skin coloration around the lips and nails (cyanosis), seek advice from an cardiac expert.

Treatments and drugs:

Treatment of Ebstein's anomaly depends on the severity of the defect and your signs and symptoms. The goal of treatment is to reduce your symptoms and avoid future complications, such as heart failure and arrhythmias. Treatments may include regular monitoring, medications, surgical heart repair (Tricuspid valve repair, Tricuspid valve replacement, closure of the atrial septal defect or ASD, Maze procedure), radiofrequency catheter ablation, heart transplantation.

Facilities available in Bangladesh:

A team lead by Dr. Maj (Rtd) M. Quamrul Islam has recently started cone repair surgery to treat Ebstein's anomaly at the National Heart Foundation Hospital & Research Institute in Dhaka, Bangladesh.

Dr. Quamrul had training on the procedure at Mayo Clinic in the USA and now he has introduced the operative procedure in Dhaka, Bangladesh.

Since Ebstein's anomaly is a rare disease, there is shortage of patients; moreover all the patients are not properly diagnosed. Some patients from the corner of the country cannot reach the right place to seek proper treatment - many of them cannot afford the treatment even. These drawbacks are the principal hindrance getting proper treatment.

Dr. Mohammad Ziaur Rahman, a member of the surgery team explained that there are various management of the disease. The first line of treatment is usually conservative treatment by medicine and monitoring. But it does not work in the long run and ultimately the patients require surgery.

Some cardiac surgeons replace the affected valve which ultimately lead to many complications and require other treatment modalities to compensate those. So one of the best treatment options is repairing the affected valve.

Repairing affected valve, which is called cone repair - requires technical skill which was not available in Bangladesh. But now it is performed by the team lead by Dr. Quamrul Islam. Already they have performed couple of cases and the patients are quite okay on follow up.

Dr. Zia told that they need more referral from other cardiologists and cardiac surgeons. It is necessary spreading the message that the procedure is now performed in Dhaka, the capital city of Bangladesh.